{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T21:46:36Z","timestamp":1789076796608,"version":"build-2803163510"},"reference-count":27,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"1","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2020,1,7]]},"abstract":"<jats:sec xml:lang=\"en\">\n            <jats:title>Background<\/jats:title>\n            <jats:p xml:lang=\"en\">\n              Information is evolving on liver disease in pediatric patients with Fontan physiology. The purpose of this investigation is to evaluate the spectrum of liver disease in a pediatric population of patients with Fontan physiology and evaluate transient elastography (\n              <jats:styled-content style=\"fixed-case\">TE<\/jats:styled-content>\n              ) as a noninvasive marker of liver disease.\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Methods and Results<\/jats:title>\n            <jats:p xml:lang=\"en\">\n              We prospectively enrolled all children with Fontan physiology. All patients underwent comprehensive liver evaluation including liver enzymes (alanine aminotransferase, aspartate aminotransferase, gamma\u2010glutamyl transferase, alkaline phosphatase), aspartate transaminase to platelet ratio index, albumin, bilirubin, international normalized ratio, complete blood cell count, abdominal ultrasound, and\n              <jats:styled-content style=\"fixed-case\">TE<\/jats:styled-content>\n              . Transjugular liver biopsies and hemodynamic measurements were performed in a subset of patients. A total of 76 children (median, 11.7; interquartile range, 8.4\u201314.8 [56% male]) were evaluated, with 17 having a transjugular liver biopsy (median 14.8\u00a0years; interquartile range, 14.3\u201317.4). All biopsies showed pathological changes. The severity of liver pathology did not correlate with\n              <jats:styled-content style=\"fixed-case\">TE<\/jats:styled-content>\n              . There was a positive correlation between\n              <jats:styled-content style=\"fixed-case\">TE<\/jats:styled-content>\n              and time since Fontan (\n              <jats:italic>R<\/jats:italic>\n              =0.42,\n              <jats:italic>P<\/jats:italic>\n              &lt;0.01), aspartate transaminase to platelet ratio index (\n              <jats:italic>R<\/jats:italic>\n              =0.29,\n              <jats:italic>P<\/jats:italic>\n              =0.02), aspartate transaminase (\n              <jats:italic>R<\/jats:italic>\n              =\u22120.42,\n              <jats:italic>P<\/jats:italic>\n              &lt;0.01), and platelets (\n              <jats:italic>R<\/jats:italic>\n              =\u22120.42,\n              <jats:italic>P<\/jats:italic>\n              &lt;0.01). Splenomegaly on abdominal ultrasound was correlated with\n              <jats:styled-content style=\"fixed-case\">TE<\/jats:styled-content>\n              (\n              <jats:italic>z<\/jats:italic>\n              =\u22122.2,\n              <jats:italic>P<\/jats:italic>\n              =0.03), low platelet count (\n              <jats:italic>z<\/jats:italic>\n              =1.9,\n              <jats:italic>P<\/jats:italic>\n              =0.05), low aspartate transaminase (\n              <jats:italic>z<\/jats:italic>\n              =1.9,\n              <jats:italic>P<\/jats:italic>\n              =0.02), and low alkaline phosphatase (\n              <jats:italic>z<\/jats:italic>\n              =2.4,\n              <jats:italic>P<\/jats:italic>\n              =0.02).\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Conclusions<\/jats:title>\n            <jats:p xml:lang=\"en\">\n              Liver disease was ubiquitous in our cohort of pediatric patients with Fontan Physiology. Given the correlation between\n              <jats:styled-content style=\"fixed-case\">TE<\/jats:styled-content>\n              and time from Fontan,\n              <jats:styled-content style=\"fixed-case\">TE<\/jats:styled-content>\n              shows potential as a prospective marker of liver pathology. However, individual measurements with\n              <jats:styled-content style=\"fixed-case\">TE<\/jats:styled-content>\n              do not correlate with the severity of pathology. Given the prevalence of liver disease in this population, protective measures of liver health as well as routine liver health surveillance should be implemented with consideration for hepatology consultation and biopsy in the event of abnormal liver biochemical markers or imaging.\n            <\/jats:p>\n          <\/jats:sec>","DOI":"10.1161\/jaha.119.012529","type":"journal-article","created":{"date-parts":[[2020,1,4]],"date-time":"2020-01-04T10:00:22Z","timestamp":1578132022000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":51,"title":["Fontan\u2010Associated Liver Disease: Spectrum of Disease in Children and Adolescents"],"prefix":"10.1161","volume":"9","author":[{"given":"Steven L.","family":"Rathgeber","sequence":"first","affiliation":[{"name":"Division of Cardiology Department of Pediatrics British Columbia Children's Hospital and University of British Columbia Vancouver British Columbia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Orlee R.","family":"Guttman","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology Department of Pediatrics British Columbia Children's Hospital and University of British Columbia Vancouver British Columbia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna F.","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine British Columbia Children's Hospital and University of British Columbia Vancouver British Columbia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christine","family":"Voss","sequence":"additional","affiliation":[{"name":"Division of Cardiology Department of Pediatrics British Columbia Children's Hospital and University of British Columbia Vancouver British Columbia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicole M.","family":"Hemphill","sequence":"additional","affiliation":[{"name":"Division of Cardiology Department of Pediatrics British Columbia Children's Hospital and University of British Columbia Vancouver British Columbia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard A.","family":"Schreiber","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology Department of Pediatrics British Columbia Children's Hospital and University of British Columbia Vancouver British Columbia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin C.","family":"Harris","sequence":"additional","affiliation":[{"name":"Division of Cardiology Department of Pediatrics British Columbia Children's Hospital and University of British Columbia Vancouver British Columbia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"e_1_3_1_2_2","doi-asserted-by":"publisher","DOI":"10.1053\/j.pcsu.2010.02.006"},{"key":"e_1_3_1_3_2","doi-asserted-by":"publisher","DOI":"10.1038\/nrcardio.2010.99"},{"key":"e_1_3_1_4_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1747-0803.2007.00116.x"},{"key":"e_1_3_1_5_2","doi-asserted-by":"publisher","DOI":"10.1053\/j.pcsu.2015.11.006"},{"key":"e_1_3_1_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2016.07.006"},{"key":"e_1_3_1_7_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-012-0315-7"},{"key":"e_1_3_1_8_2","first-page":"456","article-title":"Liver disease in patients after the Fontan operation","volume":"117","author":"Krishna K","year":"2015","unstructured":"Krishna K, Pundi KN, Kamath PS, Cetta F, Li Z, Poterucha JT, Driscoll DJ, Johnson JN. 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